The updates, stories, struggles and challenges of Aurelia Dittrich, a young girl first diagnosed Hypoplastic Left Heart Syndrome, a congenital heart defect, and then Osteopathia Striata with Cranial Sclerosis, and also Cyclical Vomiting Syndrome. This blog will also talk about the amazing support and love and journey of her older sister, Madison.

Friday, November 4, 2011

Aurelia continues to grow and thrive and is doing fantastically well, but I am getting ahead of myself. Our last update was in July, just after her heart surgery. She recovered well and we were home a week after the surgery, though there were a few difficulites along the way. She is a quick healer though. We were not supposed to give her belly time for a full two weeks after her surgery, but that didn't stop her from giving herself belly time. Prior to the surgery, she had been working on crawling (a very slow form of it that includes lots of rolling), and she wasn't willing to let a little thing like open heart surgery get in the way of her practice! The doctors told us that she would self-regulate and if it hurt her to be on her belly, she wouldn't do it. Well, either she's one tough cookie - or it really didn't hurt. She spent as much time as possible on her belly!

We were relieved that we did not have to turn the distractors in her jaw any more. That finished just prior to her heart surgery. Now the plan was to wait and allow her jawbone to heal. We returned to CHOP in late August for a follow-up sleep study and I was happy that we were able to go home the next day (quite different than the previous one that ended with a 2-week stay in the hospital!). We later got the results of the sleep study that still showed mild-moderate sleep apnea, but it was GREATLY improved. Which means that the mandibular distractors worked and that the pain and frustration of moving her jaw was worth it. There was a chance that she would have gone through all of that and it wouldn't have worked. We were ecstatic to hear that the procedure was a success! Aurelia was scheduled to get the distractors taken out in September, just 2 days before her first birthday.

While we waited for that surgery date, she continued with her weekly therapies. She has a Physical Therapist (Miss Marjorie) and a Developmental Therapist (Miss Lynne), who she was been working with since February. In July, we added Occupational Therapy (with Miss Judi), which she also gets weekly. She makes improvements daily. In July, while she was in the hospital, she began sitting up, unassisted. And now, just a couple of months later, she is army crawling and is able to get anywhere that she wants to go. Over the last week or so, she began to get herself into a sitting position from a laying position, all by herself! Aurelia really is one determined little girl, which is wonderful to see such fierce strength and will. Madison is truly her biggest cheerleader ~ she jumps and shouts out "you did it Aurelia! I'm so proud of you!" each time that Aurelia sits up!

Another addition to Aurelia's daily routine has been the introduction to FOOD. Yes, actual baby food! In August, she had an appointment with the feeding team and while they are cautious, they are allowing us to give Aurelia an ounce of stage-2 baby food one time a day. The fear is that she is a risk for aspiration, so we have to be very careful. Also, because her core isn't that strong yet, she tires out very quickly. An ounce of food isn't a whole lot, but it is about all that she can handle at this point in time. The introduction of food has been a slow one. Aurelia had been grabbing at our plates/forks/anything left close by for months. And she brings everything to her mouth, but as we discovered when we first gave her baby food, she didn't quite know what to do with the food when she got it into her mouth. Like any baby getting their first tastes, she spit most of it out. She is extremely enthusiastic though and grabs the spoon from us with almost every bite. From the first time that I gave her some applesauce, she has tried to feed herself. And I'm happy to say that now, nearly 3 1/2 months later, most of the food actually gets swallowed. AND, she's been enjoying little star puffs, so she is slowly getting the idea of how food and her mouth are supposed to work. We will reevaluate all of this once she gets her cleft palate repaired. We will likely have to start all over because she'll have a whole new mouth, which will work a bit differently. In the meantime, we soldier on with trying different tastes and different textures. In fact, we just added a FOURTH weekly therapy. She will now be seeing Miss Cindy, a Speech Therapist, on a weekly basis too. It is great to have so many services available to us! In PA, she gets all of these therapies at our house (until age 3), which is so covenient. I try to be home for all of them, but it is great that she can still have therapy at home when I can't be there (it is good for our new nanny, Kaitlyn, to see what goes on in the therapies too).

On to the surgeries... As I mentioned above, Aurelia was supposed to get the distractors out on September 21st. But, our little girl caught a cold and it was decided to postpone the surgery. No need to put any additional risks on the surgery. We had a to wait a full month, but on October 24th, the distractors FINALLY came out! Aurelia healed quickly and we were only in the hospital for one night. What a miracle worker she is!

And now we are anticipating her FINAL surgery, cleft palate repair, for next week, November 9th. This will be surgery #12 and we are so excited that it will soon be over. Again, it should be a one night hospital stay. Some kids stay longer, but since Aurelia is primarilly tube-fed, we don't have to worry about getting her back on an eating schedule. I'm sure that her mouth will be sore. In fact, one of our therapists noticed this week that Aurelia's tongue seems to be tied, so there may be an additional part to her surgery. I called her plastic surgeon, a really great guy named Dr. Jesse Taylor, and he'll take a look at her when we come in for the surgery and decide whether or not he can do that repair at that time too. I still can't believe that none of us noticed her tongue before because it is now so obvious to me. Either way, we are very hopeful that once her cleft is repaired, we'll be able to increase her oral feedings.

I hope to write more next week and try to keep this blog a bit more active with entries. I will also add some photos later, but I really wanted to get you caught up on what has been happening since July. As I said, our girl is growing and thriving and doing really well. We are so proud of the work that she is doing and with how far she has come.

Sunday, July 17, 2011

Hi folks. Sorry for the delay in posting these last few days, but I needed a break from writing. It's been a stressful day or two and I just didn't have the wherewithal to sit down at the computer and type something out. Aurelia is stable and in a similar place to where she was a few days ago. She still is terribly gassy, has needed medicine to help move her bowels, and is still watched closely for the amount she urinates. Her fevers spiked a few nights in a row, but last night it was not quite so dramatic a rise.

In terms of pain Aurelia is on oxycodone and tylenol, if she needs the additional relief. Sleep has been difficult for her, in part because of the gas, but also because she is so nosy. Our girl has to inspect everything going on in and everyone entering the pod. We hope to be out of intensive care soon so we have a shot at a private room. Her recovery should go well there.

Aurelia's breathing is still assisted by 1/4 liter of oxygen, but that is what she came in on, and she is a terribly difficult ween. We hope to start weening her off morphine and diazepam again soon, and maybe her oxygen, too. She has to relax, more, though, and breathe deeply for that to happen.

Not much to report other than that. Jillian went home for a night and returned with my in-laws and Madison as we approach this next phase in the step-down unit. I'll be returning to Gettysburg for a few days to be with the cats tomorrow morning sometime, I think. Well, thanks as always for all your support and prayers. Hope you are all well!

Friday, July 15, 2011

That's right, folks, that's the sound I make when the other shoe hits me square in the noggin. So far the other shoe appears to be something light, a ballet slipper perhaps, as opposed to a steel-toed work boot, but this is still a bit of a disheartening morning for me.

After a lovely afternoon visit yesterday from Grandma and Grandpa Dittrich, Aurelia endured a difficult evening. Her belly swelled substantially, she had trouble peeing, pooping, and she became magnificently gassy. Her pain medications failed to alleviate much discomfort and sleep eluded her for much of the night. With two fever spikes up to 101.8 we guaranteed our stay here in the CICU for at least another day.

The team took a urine analysis, blood analysis, and cultures, looking for infections that might be lurking somewhere unseen. This morning the urine analysis came back clean and Aurelia's white blood cell count is reasonable. Thankfully her fever is down after some ibuprofen and she moved her bowels and peed substantially a little while ago.

Currently, our best guess is that her intestinal track failed to start up to full speed after her surgery yesterday (remember that comment I made about her kidney seeming to have started up?), which meant that she had limited function, but not enough to tolerate full feeds. So all day yesterday, when we ran full feeds, Aurelia's body failed to fully process her food. This resulted in the gas, constipation, lack of urine, and painful belly swelling. To help her the team stopped last night's overnight feed and we will start a Pedialite feed later today to see if things are back up and running. We will keep you all posted.

Oh good news! I forgot to mention that pulmonary no longer believes Aurelia needs her inhalers. Yay!!!!

Thursday, July 14, 2011

Good news continues unabated this morning as Aurelia's improvement continues. All of her tubes and wires, other than the IV, are out and gone, her normal feeds have resumed, other than morphine and diazepam she is off pain medication, and her breathing has not gotten any worse. Our only concern thus far is a stubborn bit of fluid in her lungs. We have started chest PT and increased her diuretics in an attempt to drain her lungs more so we can ween her off oxygen.

A liberal estimate has us leaving the intensive care unit today and maybe moving into the step-down unit tomorrow. I cannot believe Aurelia is doing so well! I don't want to jinx all this progress, but I confess to feeling a bit of disbelief regarding her progress. Never has anything like this been so smooth for us, so I keep looking up for the proverbial shoe, or anvil, or whatever, to drop. I sincerely hope it doesn't, but have a hard time believing it won't!

We have a few questions for doctors on rounds today, when they make their way to Aurelia:
1. How long do we have to do wound care behind her ears?
2. Now that her tracheomalacia resolved itself, do we still need her inhalers?
3. Can we get a swallow study while we are here?
4. Can audiology follow up with Aurelia while she is here, too?

We also want to try and contact our home care company contact here and get a letter of medical necessity to help our application for medical assistance. We want to try and obtain a full time home care nurse for Aurelia and see if we qualify for medical assistance insurance, in the event our insurance starts to play hardball with CHOP and us. We received our first rejection for care a few weeks ago, so we are thinking the fights are beginning as she vaults way past the multi-million dollar baby level. We estimate her care to be at or beyond $2,000,000 since her birth in September. Combine that with 119 days of leave without pay for Jillian and we're thinking medical assistance is something we definitely need to have! We'll keep you all posted!

Wednesday, July 13, 2011

All good news from CHOP this evening, even as chaos reigns in the pods around us. With critical emergencies proliferating in the CICU Aurelia bucked the trend, enjoying a quiet afternoon of rest and healing. Her O2 saturation is wonderful at 2 liters of oxygen, she is not maxed out on her pain medication in case she needs more, her color is good and her kidney is operating wonderfully.

That last bit, about her kidney, had us particularly concerned. When you have your heart stopped and you go on heart-lung bypass there is a risk that your other organs will shut down and have a tough time starting back up again when your heart begins beating again on its own. Given that Aurelia only has one working kidney, if that doesn't fire up and work well from the get-go then she runs a risk of fluid build up and that can be dangerous.

Regardless, Aurelia is having great fluid discharge through her foley catheter and the only fluid she's needed (besides nutrients) is some potassium, to replenish what's being lost due to her lasix. Her blood gasses have been great all afternoon, drainage from her chest tube is within acceptable limits, and even her wound care went well around her distractors. All in all, it has been a super afternoon. Thank you to everyone for all your support! Jillian and I feel tremendously blessed to have such a strong and dedicated support network of friends, family, and colleagues.

We just received word that Aurelia has successfully underwent surgery. Dr. Spray repaired both her holes and augmented her pulmonary artery. He says that although her mitral valve appears slightly misshapen there is no leaking or backflush of blood across the valve, which is good, and it appears that whatever muscle bundles might have been in the way of her VSD repair have been cut away. Apparently, Aurelia's heart has become a little larger to compensate for the resistance placed against blood flow due to the PA band she received in October. As a result, the increase muscle tissue in her heart might have obstructed those areas the surgeons needed to access when repairing the VSDs.

The echo cardiogram taken immediately after surgery shows no leaking across the two chambers of the heart and no leaking across the valve, as I mentioned above. It also appears that her heart is successfully handling the full blood flow into and out of the lungs, and through her body. Whether or not her heart could handle pumping by itself is a concern, primarily due to the fact that her left ventricle sits right on the border line of a functioning/non-functioning ventricle (which is based on its capacity and ability to maintain adequate pressures and flow).

Currently, Aurelia is off the heart lung machine, being extubated, and undergoing an x-ray. We will be watching her carefully over the next day or two for effusion, or increased fluid around the heart, and any change in her heart's ability to handle blood flow to the lungs and body. Again, due to her heart's increased musculature the trick is going to be getting her heart to relax substantially, so that it can fill to complete capacity, and subsequently pump blood to and from the lungs to complete capacity. In the past her heart built up its strength and did not pump to capacity because of the restrictive PA band. Hopefully, though, given the medicine she is on we will see improvements over the next few days as her heart begins to fully manage the appropriate blood flow of her body.

Thank you to everyone for their prayers and well-wishes. These next 24 hours will be crucial in Aurelia's recovery, so we will keep you informed. For now Aurelia is out of the dark heart of the forest and onto the path, but the way is steep and overgrown, and fraught with briers and other dangers, so keep those prayers and positive thoughts coming.

God I'm so nervous. Aurelia has been in surgery for two hours now, the first being spent with anesthesia and this last having her chest open, tubes and lines placed, and getting through her scar tissue. Reports from the team are positive so far, but I cannot fathom how Aurelia endures this. I cry thinking about how strong and determined she has to be, without choice, in order to live. I would do everything and anything to take this burden from her, to let her live without this pain and trauma.

I know some people believe that young children do not remember events such as these, but I believe they do. I believe that any person, no matter how young, remembers these traumatic events and that they are imprinted, that their development as people is changed forever. I don't fear these difficulties impeding Aurelia's development and personality, but rather I think the courage and strength and happiness she shows now will only be magnified, increasing as she ages, as these traits have served her best today and so far.

When she comes back from surgery Aurelia will have a number of lines placed in her body. She will have a chest drain, an arterial line, pacing lines, foley catheter, and an IV. Her chest will hopefully be closed, packed, and covered. Our primary concern then will be two fold (if all else goes well): will Aurelia's smaller left ventricle be capable of pumping blood for her entire body without assistance and will her heart's electrical tissue be damaged, requiring a pacemaker? Unfortunately, one of her VSDs is located in and around a place where her heart's electrical tissue exists. It is possible that Dr. Spray will have to stitch in that area, and if so, it may disrupt her heart's normal rhythm.

Monday, July 11, 2011

Jillian posted this on Facebook, but I wanted to be sure we recorded it here as well.

Today feels different than most other days. With Wednesday approaching quicker and quicker, I have fears and a feeling of helplessness. I am looking forward to the surgery being over with for many reasons. Aurelia’s heart will be whole. Complete. Healed. For that I am thankful to the hundreds of doctors, nurses, surgeons, and others who will have taken part in her care. Today’s feelings aren’t about the upcoming surgery though; they focus more-so on our daughter, Madison, who has been such a trooper through all of this. Don’t get me wrong, Aurelia is my little heart warrior and I think that she is the bravest person that I know. Madison though, has been through every bit of this with us. And she has weathered it all very well. I am very proud of the caring, sweet girl that she has become. She is the best big-sister in the world and I absolutely treasure her. I am looking forward to normalcy in our family. Of the last 10 months, I have spent 116 nights away from home, at three different Ronald McDonald Houses. That is nearly 1/3 of a year. This next stay will likely be a week or two and this one I am dreading the most. Madison has been with me for nearly every night for the last year, but this time Charley and I are opting to leave her at home with my parents taking care of her. But leaving her behind is tearing me apart. I know that it will be better for all of us in the long run. Charley and I can spend all of our time at the hospital with Aurelia, especially during the first few days post-op. We won’t have to rush back to the RMH for dinner or worry that we aren’t splitting our time equitably between both girls. For Madison though, hopefully it will be a time for her to enjoy time at home with her grandparents. She can play in her pool or in the sprinkler and have all of her toys and books close by. She can sleep in her own bed. She will have her Uly and Shy (our cats) and she will have time for arts and crafts and the undivided attention of her grandparents. For my part though, I am going to miss her dearly. Having her with me for those first 11 weeks of Aurelia’s life (and the week before, when we went to Philly early) was such a blessing. She was a wonderful distraction on tough days. It was good to go “home” in the evenings and have her to be with, especially when Charley wasn’t there. I think that I was able to be with her more and to see her grow and change more than I would have if I had been working all of that time. It really was very special. But this time, I think that we need to focus on Aurelia and to spend our time and energy on her. I guess that anyone that has more than one child feels torn when they can’t be with both kids at the same time. This just feels so painful though. And while there are still 2 surgeries ahead, I don’t have the same sense of angst when thinking about them. Maybe that is because they are not right in front of me yet. In the grand scheme of things, they will be minor compared to many of the other surgeries that Aurelia has already faced. They will also be much shorter stays in the hospital, and hopefully they won’t have the risk of as many complications as are possible with this upcoming surgery. No matter what, right now I am feeling scared about the next two weeks. I would love for Aurelia’s surgery and recovery to be as smooth as possible, but I know our girl and there is bound to be some sort of a complication. I take solace in knowing that Madison is just a car ride away. And perhaps when Aurelia is stable, I’ll go home for a night. Or maybe bring her back to Philly. After all, I’m sure that she will be missing her sister (and her Mama and Daddy) just as much as we’ll be missing her.

Thursday, July 7, 2011

I'm sure I've used that title before, but man am I tired. It has been a whirlwind these last three days. Aurelia and Madison are adopting to life at home again, and for the most part that's going smoothly. Aurelia does her best to be good natured even as we turn screws in her jaw every day, disinfect and slather up her ears in ointment. Madison is coming around to once again be the girl we knew in April and May, but this is the first time I've seen her adaptation slowed somewhat by jealousy. Her needs have visibly intensified for the first time since this odyssey began.

Our poor cat Ulysses had a serious infection on his hindquarters. Due to stress, we hope, he started rubbing his ass along the rug while we were gone. He rubbed his skin raw. He was a bloody, swollen, infected mess. Now he's still swollen, but he's shaved, bathed three times a day (which he loves), and instead of bloody he's all covered in a sheen of ointment. Yes, that's right, I get to rub ointment all over my cat's privates, including his butt. Be jealous. In the pity category, he is a cone head kitty for a few weeks and has to stomach the indignity of being given two different medicines throughout the day. That's a fun time for everyone, let me assure you.

It has been an interesting time for me and Jillian as caretakers during this week long hospital respite. Aurelia's care schedule is so complicated and covers so many medicines with changing dosages each day that we had to make up medicine and care charts, different ones for each day, so that we can be sure she comes off her pain medication comfortably. It's been challenging to balance those needs (which are frequent), Uly's needs (less frequent), and Madison's increasing needs (less frequent still, but more than ever before), with our needs to catch up with and take care of everything here at the house from our recent stay away.

Now that we are on the second half of the week and moving toward our next stay at CHOP things are beginning to shift into planning mode for our time gone. We think we'll have Madison stay here at the house with my in-laws to reduce the burden of travel on her. We are also thinking about how we'll handle Ulysses and the other household issues that my in-laws would have to take care of while we're gone; we are appreciative of their help, but we certainly don't want to abuse their good will!

Monday, July 4, 2011

Note: I wrote this yesterday in my diary, only now am I getting it posted.
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Ah piss. Aurelia continues to improve and I continue to worry. Our girl begins to sit on her own, even in a hospital bed, and I freak out over her needing a 1/4 liter of oxygen at night to sleep. Everything, all my fears, are based off of past experience. It seems every time we approach discharge something happens that may delay our leaving.

This morning we arrived at the hospital to find Aurelia with bandages over her ears. It appears that some sort of infection has settled in over her distractor sites and we are waiting to see what, if anything, will come of it. We have, too, begun the usual dance around Aurelia and her oxygen. As such a difficult ween, it seems we may convince the doctors to send us home on oxygen - only if her ears look O.K.

Still, I do believe they will let us go home tomorrow or Tuesday. Even a little break would be so very welcome.

Saturday, July 2, 2011

We have made our way to the step-down unit in CHOP. In brief, Aurelia chugs along, improving with each passing day. Her pain medication woes lessen, her strength increases, and her oxygen doses decrease over time. Today she's needed no additional tylenol or ibuprofen, she is down to 1/2 a liter of oxygen, and she managed to sit upright on her own with limited support for nearly a minute. All these are wonderful milestones.

We are pushing the doctors to let us go home for even a bit before her next surgery on the 13th. We have to return here on the 12th as it is, so any time at all would be welcome. Some sense of normalcy brings with it amazing relief, even for a few days. I imagine we will be back here for two weeks more starting on the 12th, so I pray for a break soon. Our family was together at home for only a week after school ended for me before we traveled to Connecticut and found ourselves here two weeks ago.

Let me say thank you to so many of you who have supported us; your facebook messages, blog comments, phone calls, caring for our cats and one helluva hardy fish at our house in Gettysburg, and the countless prayers, positive thoughts, and well-wishes have sustained us mightily. One day last week more than 100 people read our blog, sending their good thoughts and prayers Aurelia's way, and for that we say 'thank you.'

It has been brought home to us time and again, this most recent stay no exception, that our employers, the Northeastern School District and Carroll County Public Library System, are amazingly compassionate. Colleagues have sent cards and gifts, offered help and support, and have made themselves available to us in this, and all our other, times of need. Thank you. You have helped raise our daughter and have made sure that Aurelia not only lives, but lives well and strong. You are a part of her family and we look forward to the time she meets you all.

I want to add, too, that so many of you have asked after our other daughter, Madison. Although only two, in the last year and a half she's had live-in house guests for nearly twelve weeks when my wife was on bed-rest with Aurelia, she has had to live away from home in a hotel room for nearly four of the last ten months, and she spent nearly three months with her dad two hours away in Gettysburg, as I had to work immediately after Aurelia's birth. Madison endures with great poise, and determination, and joy. Such pure, happy joy as only a two year old can have. She has been a gift to all of us, helping us laugh and smile, and encouraging us to always remember that family, no matter the challenge it endures, is most important. You have all helped raise her as well as Aurelia, in your own way. Thank you.

Thursday, June 30, 2011

Strange is the word I use to describe the previous 36 hours. Aurelia's condition steadily improves, but at the same time managing her care becomes increasingly difficult. Since last writing, Aurelia lost her breathing tube, ended all IV pain medications (minus a small dose of morphine through her g-tube), resumed normal feeds, lost her central line, avoided any fevers, and is weening off oxygen.

I should jump for joy! At the same time, this is no three month old largely devoid of personality; Aurelia is nine months now and tremendously capable of self-expression. Discomfort, impatience, pain, exhaustion, irritability, all these and more our girl now shares, vociferously, with entire pod of babies.

Let me clarify. As Aurelia came off her breathing tube she expressed joy, but suffers irritably from a terrible cough and partially collapsed left lung (not something unusual - it often occurs in little ones who are kept sedated for some time). Angrily, Aurelia fights against her high flow nasal canula, the treatment for her lung problems. Fed up as she was with the breathing tube, the canula must feel like torture - she had this big tube removed, she fought to breathe on her own, and now they are forcing even more air into her lungs.

Now that the majority of her pain medication has stopped, Aurelia's color and personality return vigorously, but with that comes increased jaw sensitivity and discomfort in her leg from her central line. I would almost trade away the smiles that arrive as her pain medication stops, especially if it meant no longer watching her twitch, shake, and moan constantly from narcotic withdrawal. For some reason watching her filled with addiction fueled agitation and ache breaks my heart near as much as seeing her sedated into a stupor.

And if any of you know an addict, you know how terribly difficult it can be to rest. Aurelia has managed only six hours of sleep in the last 36 and that came thanks to diazepam's ability to take the edge off her craving. Hopefully, the combination of getting her central line out and maximum doses of ibuprofen will give her some rest.

Of course I know that this is all preferable to where she was four days ago, but seeing her so distressed, watching those emotions play across her face, it breaks my heart in ways surgery and long term stay hasn't in the past. Here's to hope! Maybe the step down unit soon. Maybe a chance at getting home, even for a few days, before we have to return for her open heart surgery. It's possible!

We had another interesting evening here in Philadelphia. Unfortunately Aurelia's last IV infiltrated and we had already left for the day. Jillian, Madison, and her mother spent the night in Gettysburg, much like I did last week, caring for the cats, cleaning up from lack of use, and doing some shopping. Dr. Davies, the fellow on duty, called at midnight to tell me that unless a viable vein could be found we would have to place a central line.

Those lines enter an artery at the groin and, while more permanent than an IV, are still less i9nvasive than a PICC line. I gave my consent, knowing that the procedure itself is fairly low risk, and today she looks far more comfortable.

Medicinally, Aurelia is down to 1.5 mcg/hr of Dexmedetomine and 2mcg/hr of Fentanyl, both steps in the right direction as we move toward extubation. Our plan is still to extubate today, and her nurse, Jessica, is weening Aurelia off the ventilator. Aurelia's temperature is fine, or low grade, as she wrestles with whatever it is she has in her lungs.

I'm hopeful today. Maybe we will have this tube out of our girl's lungs and we can get down to the business of accurate and adequate pain management for her distractors. Ideally I want plastics confident enough in Aurelia's status to train Jillian and me to move her jaw and thereby release us before we have Aurelia's open heart surgery. There is a chance they will want to keep us here until then, anyway, and that frightens me terribly. One day at a time, though, so we'll see.

Monday, June 27, 2011

You can tell time with hospital monitors. Sudden beeps, chirping machines, and intermittent cries mark milestones, victories, achievements, and setbacks alike. Nurses come and go with the changing of the guard and every so often doctors appear to mark the noon and midnight of hospital life, rounds. Parents greet the day like so many commoners swept away in tidal events beyond their control, pulling with or pushing against, the rushing daily currents.

So much is beyond your control as a common cardiac parent. Every day is toil; exhausting, unyielding trudging toward whatever end. Aurelia is a warrior amid this struggle and her care team the aristocracy, grand and minor nobles alike, but we parents are commoners. We search for whatever ways we can to better our lot and the lot of our daughter in a world largely beyond our control. Maybe it's through an observation we share with the care team, or some piece of knowledge gleaned from experience as her day to day caretakers, or even questions we ask to keep ourselves and everyone else on their toes.

We scramble around like this out of love, of course, but also to fight off fear and its potential powerlessness. This is a good and worthy struggle, but it can make you so tired. Everything slips behind an omnipresent haze, clouding conversations, thoughts, even feelings. Some days little can clear away that haze except for the passing of time, one nurse, doctor, beep, or cry, at a time.

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Today Aurelia made some progress, baby steps size, but progress nonetheless. Her labs came back mostly clean, so she's off contact separation and whatever she does have brewing in her lungs is so not concerning that folks are going to let her fight it off on her own. Her fever is not sticking - if it shoes up, it goes away again with some tylenol (and even that is not always needed) - and she continues to tolerate her jaw movements reasonably well.

The tough part about that is avoiding any jiggling or jostling of the screws while attaching the screw driver. Her jaw is still mighty sore, so attaching that to her can be the hardest part of the job. We are slowly weening her off the respiratory machine and we hope to extubate her sometime tomorrow. I'm very excited about that as Aurelia will no longer need to be restrained. Watching her flail about because her arms are tied down to the crib is heartbreaking. Still, she is so brave, and so strong, and so determined. When she's awake now it isn't to have those silent screams every time, it's to make eye contact and to furrow her brow and to blink, as if to express her will and effort to overcome this struggle. Or, more likely, she's trying to fart. She's been really gassy lately.

Speaking of which, she's only had two bowel movements in over a week. We're not worried, but man, you ever seen a 17 lb. baby unleash a breast milk diaper? I swear to you that my time in Purgatory is greatly reduced because of what we've seen, smelled, and cleaned. To that end, in an effort to be sure all of Aurelia's insides are working right we did a hypoglycemia test today, hoping to see if her retching at the end of meals is related to a drop in blood sugar. We'll keep you posted. We'll also be doing a hearing test to dot some 'i's" and cross some "t's" with our doctor. I'm inserting a picture below of her jaw apparatus, via x-ray, so you can get a clear understanding of how this all works.

Here you can see a number of things on, around, and in Aurelia. From the bottom up: You can see the wires wrapped around her sternum from her previous open heart. Just above that you will see some of the sensor wires on the outside of her skin, wrapping around her torso. Above that are two separate marks on the x-ray; to the right is the band around her pulmonary artery, restricting the blood flow to her heart. On the left, moving up her throat from that location, is the breathing tube intubated through her mouth. That brings me to her jaw. You can clearly see the mandible device attached to her jaw in two places on each side. Between those two anchor points is the incision that splits her lower jaw. As you follow the metal bracings up behind her ears, you see the two screws that emerge from her skin. As we turn those with a special screw driver, the jaw splits along the seam between the anchors, moving the lower portion further forward and bringing the tongue along with it at the same time. This will help her breathe by enlarging her airway.

And she looks like a bad-ass combination of Skeletor, Megatron, and the Terminator. My daughter is a ass kicking cybernetically enhanced warrior. Bring it on, open heart surgery! I HAVE STEEL JAWS!

Sunday, June 26, 2011

It was not a good night. After attempting to lower Aurelia's pain medication to give her a little more consciousness as we move toward removing her intubation, we had a serious setback at about 3:00 this morning. During a diaper change Aurelia used ninja reflexes to yank her sutured breathing tube out of her nose. As the team scrambled Aurelia held her own for a few minutes before crashing. Her heart rate and oxygenation plummeted and the team had to do chest compression for 3.5 minutes as they attempted to circulate a variety of medicines through her body.

Aurelia did not lose her pulse or stop breathing, but in order for the paralytic and stimulants for her heart and lung to reach the important locations the team needed to be sure her body kept its blood and oxygen moving. Once paralyzed they picked her up to a standing position and intubated her through her mouth. Things became more complicated when she did not come out of her medicated paralysis the way everyone hoped, so we stopped all pain medication, using the pain to stimulate her body into moving again. As of now, we are back at 1/2 the dose of pain medications we were using yesterday.

Of course this morning Aurelia lost her remaining IV and needed to get a new one in her right leg. She also has a fever again and we drew labs and sent off some of her phlegm to be cultured. There is some thought that an infection could be causing Aurelia's difficulty breathing without assistance. I personally believe this is a reflection of her typical difficulty coming off oxygen. When she had pneumonia this spring we needed to keep her on 1/32 a L of oxygen for a week just to keep her airway open. That's nothing - it's like a whisp of air that barely does anything. Aurelia has always taken a tougher track when it comes to breathing on her own. Another thought could be swelling from her adenoidectomy. We'll see. Either way, it doesn't look like we will be removing her breathing tube tomorrow. I hope only for a good response from the labs and culture so that we can just worry about getting this tube out of her throat.

She's got a bath now, new linens, and she finally had a bowel movement, so here is to a day of no problems or troubles.

Here's our girl after her rough night. You can see the tube now taped down into her mouth as opposed to her nose, you can see her right arm restraint, keeping her from yanking at the tube, her new IV line behind the brace on her right leg, and a fresh diaper.

Saturday, June 25, 2011

Today has been a relatively quiet day. Aurelia went through the night with only a few hiccups, trying to turn over a few times, needing a pain management rescue now and then, and otherwise attempting to wiggle free of her intubation. Truly, though, she's doing relatively well. She's a wonderful fighter determined to beat all that comes her way. She inspires Jillian and me to keep faith and strong together.

Plastics came by this morning and moved her jaw two millimeters. Aurelia took it on the chin like a champ (da da dun, ching!) even after only 30 minutes lead in time with a pain med treatment. She did so well the plastics folks are intending to come back tonight for another 2 millimeter extension.

The day itself has been rather uneventful which oddly keeps me on edge more than a crisis would. Aurelia had some mild alterations in her pain medications to keep her sedated and from turning over (which she successfully completed early this a.m.) and subsequently aggravating her jaw. The IV she lost yesterday in her foot has yet to be replaced because they taped down and isolated the remaining IV in her left arm with a brace. We hope her veins are repairing themselves in case we need to drop a second line in for medicine administration should this last IV infiltrate.

Aside from that small adjustment to her pain medication, our daughter continues to gain strength. Her oxygen saturation levels are good, no longer suffering from momentary desaturation and she appears to be bouncing back well. We have a few concerns, such as a stubbornly resistant slight temperature, a fairly good amount of phlegm coming up from her lungs via suction, and a resulting junky sound over the stethoscope. The attending isn't terribly concerned; fevers are common after surgery and broken bones, and Aurelia's labs yesterday came back great. Similarly, if there is an infection there have been no substantial changes to Aurelia's vitals, meaning she is resisting the bacteria/virus well on her own. If there is a change in any vitals then we'll culture some of the phlegm from her lungs and draw more labs.

We are hoping for some more good news soon, however, particularly regarding her tracheomalacia. ENT did not mark malacia as present during the scope, so we don't know for sure, but we can hope that our little warrior has outgrown this airway debilitating problem. On a scale of 1 - 4, with 4 being, "HOLY CRAP, CALL IN THE REINFORCEMENTS," Aurelia's throat is listed as a 1, or a non-critical airway. That thrills us, since six months ago she'd have airway collapses from getting too worked up! We look forward to final confirmation from ENT, but we think the news will be good. We hope, anyway.

So that's all we have for now from CHOP. Maybe more later, if not, definitely more tomorrow.

Here's our girl this morning, resting after having her jaw advanced. Please no comments about her thighs. She's very sensitive about them. ;)

Friday, June 24, 2011

Our time in the CICU has been full of memories, good and stressful, as we see old faces and remember previous pod-mates during our past stays. Our attending is Dr. Naim, plastics is led by Dr. Taylor, Dr. Rizzi worked on her from ENT, and even her cardiac anesthesiologist has been someone we know from previous operations. All these people help Jillian and me feel at home here since everyone has a history treating Aurelia.

Dr. Naim in particular is delightful; she cared for Aurelia during our daughter's first few weeks of life and has remarkable background knowledge about our little one's needs. She so adores Aurelia that she requested her as a patient upon our admittance and routinely comes crib side just to 'get some love' from our daughter, who smiles and asks for hugs from all who come near. Dr. Taylor continues to impress us with his knowledge and affable nature, and Dr. Rizzi's bedside manner always keeps us at ease.

These are the miracle makers at CHOP. They have made this entire task seem remarkably less daunting, especially since we are looking at three very difficult months - three weeks of pain inducing jaw advancement (what is in essence daily resetting of a broken jaw, combined with additional bone growth), the subsequent final healing of her jaw and neck, open heart surgery, healing, and hopeful improvement.

Today Aurelia is much the same as yesterday. Three times she's tried to roll over (not an easy task with your breathing tube shoved down your throat and sutured to your nose) and other than that we need to keep the status quo - sedated and comfortable, not too riled up, work her feeds back up to normal amount, decrease her breast milk fortification while she's not moving around, watch for infection, and monitor her lungs and jaw.

Today's x-ray came back looking good, no signs of anything unexpected, her swelling is reasonable, and it appears we will leave her intubated throughout the weekend. Even though Aurelia is not listed as a critical airway, plastics is hesitant to extubate our girl over the weekend when fewer staff is on duty at the hospital. We won't start moving her jaw today, but we will probably start tomorrow with 2 mm increments. This is an increase from 1 mm, but Dr. Taylor believes Aurelia can handle the increase. The plan is to move her jaw and keep x-raying her until we are comfortable with tongue placement inside Aurelia's mouth, relevant to the back of her throat. I'm terribly scared of the pain this might cause Aurelia. I think I fear this process more than anything else because it will last so long; even heart surgery's pain lasts roughly four days before it can be managed by tylenol; not this....this is something that could be hurting Aurelia for weeks on end.

The ear tubes went in without problems yesterday as well, and we are putting drops in her ears every now and then to prevent infection. She's not having any trouble with those drops because, well, she's unconscious. The only other thing we are waiting to see is the progress on her trachea malacia. Hopefully there has been improvement, but we are unsure of just what's going on with that as of this point and time. Our care team is going to get the report from ENT and then we will pass that along as we find the information.

Here is our baby girl just after surgery. You can see her breathing tube up on that contraption to prevent her from having anything on her sore and swollen face.

Thursday, June 23, 2011

So. Here we are. I wonder why it is this blog only ever sees attention when I have difficult things to post? We came here Sunday night from Connecticut (after visiting parents on both sides) to participate in a sleep study. We wanted to see if Aurelia had sleep apnea. The test revealed severe apnea, to the point of her oxygen saturation dropping thirty to forty points into the 50s. The results astounded us, making me question my ability to care for Aurelia at home; how could I have overlooked something this dangerous? We gave her oxygen, measured her saturation rates and even monitored her O2 levels daily, so how did we miss sleep apnea this bad?

Little did we know, but babies with terrible apnea often learn to position their bodies optimally for improved breathing. Aurelia's preferred sleeping position was on her side, 3/4 turned, back arched, head back. She subconsciously moved her tongue forward in her palette and minimized the impact of enlarged adenoids every night. During the sleep test, wrapped as she was and forced to lay flat on her back, she sounded like a freight train rumbling on decrepit tracks, squealing under heavy load.

Since 3:00 a.m. that morning we've been reacting to constantly changing circumstances, expectations, assistance, and fears. Monday came and went without any surgery and only the beginnings of a plan to treat Aurelia and Tuesday brought more frustration as plastics, ENT, and cardiology attempted to develop a care plan for our little girl. We did our best as a family to stick together though we all felt completely helpless and unable to control any part of the situation. Jillian and I tried to get organized for what could be a multi-week stay at CHOP, and Madison persevered valiantly, trying to have fun and understand why her sister was so sick and we couldn't go home to see our cats, Ulysses and Shiloh.

Frantically, we canceled therapy and doctor's appointments for this week, made arrangements for friends to care for our cats, secured lodging at the Ronald McDonald House, and convinced Jillian's parents to come down for a few days during and after Aurelia's surgery. We completed these tasks amid ever changing plans for Aurelia. Doctors informed us that Aurelia's care could require weeks at the hospital, that we'd be having one surgery, or two surgeries, maybe repairing her cleft palette, maybe not, maybe looking at a tracheotomy, maybe removing her tonsils, maybe doing nothing, maybe moving her jaw. What registered clearly for us was that until these breathing treatments were resolved, Aurelia would not have her next heart surgery in July.

We trust the care teams at CHOP with our daughter's life, so really we knew that our preparations had to be for the most severe scenario - a two to three week recovery from multiple surgeries leading straight into her next open heart surgery in mid-July, with us getting home sometime right around the beginning of August. I cried, cursing how a stupid sleep test could turn into more than a month away from home. I fumed, infuriated that my family was thrown into tumult with little I could do to restore balance and order.

To make Aurelia's (and Jillian's) time in the cardiac intensive care unit somewhat easier - remember that these rooms are four pods a piece, with limited family members allowed bedside by each patient, I left Philadelphia with Madison Tuesday. To her credit, Madison rallied her spirits here wonderfully, but she is just a bit too loud for promoting appropriate care in an intensive care unit. At home I could both occupy Madison and take care of some loose ends at the house. Jillian remained with Aurelia, focusing her care entirely on our little one as surgery approached.

We went into surgery this morning with a plan in place. Aurelia would have tubes inserted into her ears, her throat and bronchial tubes scoped, her vocal chord paresis examined, her enlarged adenoids trimmed down, and her mandible advanced. The surgery would involve both ENT and Plastics, and last for several hours. Aurelia has come through that surgery wonderfully and now recovers. In future posts, I will talk more about the procedures and some of the people working on Aurelia during this most recent stay here at CHOP.

Sunday, March 27, 2011

Aurelia is doing well today. She’s moving around much more than she has in nearly two weeks, rolling from side to side, and the frequency with which she’s endured suctioning has decreased as well. The latest time respiratory visited for her treatments they didn’t need to clear her airway at all! That is very good news!

For now the task is to ease Aurelia off oxygen. For most kids this is a relatively simple procedure, but with tracheomalacia Aurelia has a bit more of a challenge. They have taken her off oxygen twice so far and her SATs drop into the mid ‘80s. I’m guessing we’ll be here another day or two barring any setbacks. Aurelia’s airways will have to be clean and rested before she’s able to gather the strength necessary to breathe on her own. But, that’s what happens when your tracheal cartilage is a little too weak to keep things open.

Still, all things considered the last two days have been great. Aurelia is definitely doing better and our family is enduring life at the hospital tolerably well. I still wonder what it is about hospitals that makes you so darned tired. I’m wondering if there is a psychological study in the making here!

Saturday, March 26, 2011

It's been several days since we posted and Aurelia endured ups and downs during that time. She got past her cold, and the flu, with relatively little fanfare. In the days since Jillian's last post her parents both stayed behind and watched over Aurelia's recovery with us until Wednesday. Jillian and I made a good transition once they left, balancing all these changes well and moving into the Ronald McDonald House here in Hershey.

What a lovely facility. The people there are very nice, the house is much less restrictive, and it is right across the street from the hospital. I feel very welcome there, and the house is in terrific shape. Just being this close to Aurelia has been a boon for our family, allowing one of us to sleep with Madison close by (on tempurpedic mattresses, no less), while the other keeps vigil with Aurelia. My work is only thirty to forty minutes away, allowing me to take only one or two days off here or there, so I don't have to burn sick days needed for Aurelia's anticipated second surgery, and being so close has made us much more comfortable confronting any deficiencies in Aurelia's care.

The hospital itself, with the exception of a few days in the middle of the week, has done a nice job caring for Aurelia. We've only encountered one or two bumbling/disengaged nurses, and after a particularly bad night a frank conversation with the attending increased communication between her care team and us as parents. The night in question saw Aurelia (who had been moved out to the general pediatric floor from their intermediate care unit) have her feeds not only delayed by hours, but wasted, spilled onto the floor by a nurse who had more concern for the online course she was taking than getting a feeding tube connected correctly. Add to this an increasingly difficult couple of hours where her need for deep suctioning (where they stick a tube down your nose into your lungs) increased so much that every thirty minutes nurses had to intervene to improve oxygen saturation rates, and what we had was a girl who needed 1 liter of oxygen just to maintain baseline oxygen levels.

We found ourselves in the intermediate care wing again, waiting in a little pod for a room, at 3:30 a.m., with our possessions (and a cot) scattered around us. With a fever spiking, Aurelia retching on phlegm, and my temper fuming, we couldn't even find an attending, let alone a resident. Needless to say, Jillian and I were more than slightly distressed. Jillian took over for me the next morning and did a great job straightening out the staff here, and ever since Aurelia's care has improved, even though the diagnosis worsened.

Aurelia has been diagnosed with pneumonia. The first twenty four hours did not go well. Antibiotics started late, the chest x-ray took hours to schedule, and we had serious concerns that the course of action to heal our baby girl had been derailed by poor attention to detail. After their conversation with Jillian, however, things turned around sharply. Aurelia has been on the mend since, her fever holding steady, the garbage in her lungs coming up with increasingly less invasive suctioning, and her mood improving drastically.

She's sleeping well, smiling, laughing, and doing great for a girl that has had a serious cold, flu, and now pneumonia over the last three to four weeks. We expect to be here several days yet, barring any set backs, and we'll keep you posted.

Thank you all for your prayers and good wishes - they mean the world to us!

Sunday, March 20, 2011

It was a good day today. Aurelia was in good spirits for most of the day. She was awake for a lot of the morning and was happy to be playing with some of her familiar toys from home. She particularly loves her moose and her elephant, pulling each toward her, and often toward her mouth! I arrived with my parents and Madison around 11 am. Maddie was so excited to Aurelia she practically jumped into A's crib! She had picked out a lamb stuffed animal for Aurelia yesterday and was thrilled to give it to her. Aurelia even smiled and seemed to be pleased with her new toy. I think that she was most pleased to see her big sister! It was nice to see them together! Maddie has been a real trooper. We brought a suitcase of things for her to do and she spent quite a bit of time reading and playing with her magnadoodle. She is not nervous at all around Aurelia, which is a big difference since the last time Little A had a nasal canula. It really is a pleasure to see how much she loves her baby sister. When Aurelia gets fussy, Maddie gently pats her arm and says, "Calm down, Baby A. Calm down." It is so very sweet.

Aurelia is still receiving breathing treatments every four hours. She hates getting them, but she is so much happier after they are done. She gets a good suctioning right before and so the treatment works much better. She seems to have a fussy period each night between 7 and 8 or 9. Once she got her treatment tonight, she conked out almost immediately after receiving it! She is sleeping peacefully now.

I don't want to sound like a big complainer, but this has been a tough weekend for us (in addition to everything going on with Aurelia, Charley's grandfather passed away early Friday morning) since Charley and I have spent less than 15 minutes together alone since Friday night. It is hard to debrief as a couple when you can't actually spend any quality time together. It is a blessing that my parents were visiting and that they could help us with Madison (in fact, my mom is going to extend her stay so that she can help with Maddie in the mornings and evenings), but it is still hard.

I'm exhausted tonight and would love to write more, but I think that getting some sleep is the best thing to do. Let's hope that it is a quiet night too!

And a good morning to everyone from Chocolatetown, USA. Here in Hershey we had an uneventful night punctuated with some PT and suction, and just a bit of tylenol. Aurelia did well last night, her SATs dropping nowhere near to the level they had the night before, and other than a return of her fever (which was very borderline) she made steady improvements.

This morning she is in fine spirits, playing with her toys in her crib, and making friends with today's nursing and doctor teams. We are still in the step down unit and we will likely be here for two more days at least. At rounds this morning we discovered that the rhinovirus she has is going around and giving kids a lot of trouble, setting some up for other infections like pneumonia and RSV if not treated quickly. This made us feel good, obviously, and somewhat vindicated as well. It's hard for people to grasp that even the common cold (what the rhinovirus usually causes) can end up creating situations terribly troubling for CHD kids.

So Aurelia continues to fight, and with a smile while she's at it. The discharge plan for Aurelia will come only after her lungs are fully clear and her secretions drop, and there is no fever. So, at minimum today and tomorrow, maybe longer. Jillian and I will be working to make plans for the week, so we can be sure that we are here to take care of our Sweet Pea.

Saturday, March 19, 2011

We just heard that Aurelia’s cultures came back positive for two viral infections, rhino and influenza b. The rhino is a relief because its symptoms are similar to RSV, and her flu virus is not particularly troubling. she’s doing well with her feeds and fluids, has had only one or two instances of loose stool since yesterday, and she has kept her spirits up wonderfully well.

I have been given the all clear to hold her at will, as long as her SATs hold up, and she’s done great so far. They were able to drop her down to barely any oxygen assistance, but they’ll keep it in, I think, until absolutely sure she can keep her airway open. They are still suctioning out a tremendous amount of fluid from her nose and back of her throat, and when she coughs her chest sounds like marbles crunching on broken pavement.

My guess is we will be here for a few more days, still, but the doctors may have different plans. We’ll see what news rounds brings.

So Jillian and I haven’t written in here in a very long time. In some ways we’ve been avoiding this blog – it has a lot of painful and hard memories associated with it, and I think we feared writing in here as well; at least I know I did. To me, writing in here meant that I was at CHOP and Aurelia was not well.
I remember the joy Jillian and I felt when we celebrated our ‘Home” day, where we had been home with Aurelia longer than she was admitted at CHOP. I remember all the times where we looked at each other and one of us said, “We really need to do a blog post.” I remember, too, feeling reluctant about writing anything, because I was at home and that blog was about some mythical past where I was at the hospital.
Well, we’re back at the hospital again, and once more writing for a wider audience. Aurelia has had a series of colds for the last two to three weeks and a few days ago she developed a particularly nasty cough. This worsened until yesterday when she developed a low grade fever. Being a CHD baby with a constellation of other issues the safe and prudent bet was the Hershey ER. We convinced them not to send an ambulance (we were both getting very nervous about this first visit, even though we know that these stays will be all too frequent), and even though we both knew that we’d be admitted, we secretly hoped for a lookover and then home with daily trips for observation.
No such luck. We’ll be here for at least one more day, but I suspect longer. Truly, I’m thinking we’ll be here at the hospital until Aurelia is fully clear of her nasty cough. She’s receiving deep suction, PT, and regular breathing treatments. She’s also on a nasal canula which they attempt to wean throughout the day (with no success). Other than that, it’s life as much as normal allows.
So far we’ve only had to have one chest x-ray, and we’re suspecting an echo at some point today. Thankfully our CHOP cardiologist has an office in Harrisburg, so it’s easy to keep them updated. I’m guessing that Monday will be spent updating the CHOP teams with all that’s currently going on here at Hershey.
The folks here have been wonderful, and very attentive. I’m so thankful for the CHOP parent binder that has so many of Aurelia’s summaries and information inside; it’s been a blessing having that information at our fingertips.
I’m surprised, too, at how quickly our hospital lexicon emerged for this latest stay. I suppose having a CHD child is a bit like riding a medical bicycle where you never really lose what’ you’ve learned.
At any rate, we’ll keep you posted throughout the day, and maybe we’ll get over our reluctance to writing and maintain this blog once we leave here as well.